Clinical: Bullous Disease Flashcards Preview

Derm Exam 1 > Clinical: Bullous Disease > Flashcards

Flashcards in Clinical: Bullous Disease Deck (24):
1

Pathogenesis of the Immunobullous diseases.

Autoantibodies against skin proteins.

2

Pathogenesis of Pemphigus Vulgaris (PV)

Antibodies mainly against Desmoglein 3 (interkeratinocyte protein)

3

Pathogenesis of Pemphigus Foliaceous (PF)

Antibodies against Desmoglein 1 (interkeratinocyte protein)

4

Differentiating factor of P. Vulgaris vs. Foliaceous on PE

Vulgaris has oral involvement
Foliaceous does not
(also foliaceous looks more eroded thru the skin)

5

Modality to diagnose Pemphigus diseases.

H&E stain of epidermis
PV: lesional gap between the stratum basalis and the other epidermal layers
PF: stratum corneum shows a focal spot of thinning (erosion)

6

First line treatment for PV and PF.

Systemic Corticosteroids

7

Trial monoclonal Ab drug used for PV and PF.

Rituximab
MOA: anti-CD20 antibody

8

Pemphigus condition associated with Non-Hodgkins lymphoma.

Paraneoplastic Pemphigus

9

Pathogenesis of Pemphigoid Diseases

Auto Abs against proteins in the dermoepidermal junction

10

Pathogenesis of Bullous Pemphigoid

Abs against Bullous Pemphigoid Antigen 1 and 2

11

How are pemphigoid lesions differentiated from pemphigus lesions?

Pemphigus lesions are more flaccid bullae that rupture and because they are more superficial, less scarring occurs.

Pemphigoid lesions are more tense bullae that don't rupture as often but usually scar. Pemphigoid looks much worse than it is and usually can go into remission without treatment

12

First line treatment for Bullous Pemphigoid

Systemic Corticosteroids

13

Pathogenesis of Mucous Membrane Pemphigoid

Antibodies against:
-beta 4 integrin
-alpha 6 integrin
-BPAg2 (Bullous Pemphigus Antigen)
-Laminin 332
(eye and esophageal ulcers are common)

14

Treatment for the two types of Mucous Membrane Pemphigoid

Non-scarring: topical corticosteroids, anti-inflammatory meds

Scarring: systemic corticosteroids/cyclophosphamide combo

15

Serous sequelae of Mucous Membrane Pemphigoid

Blindness
Esophageal stenosis
Laryngeal Stensosis

16

Pathogenesis of Epidermolysis Bullosa Acquisita

Antibodies against Type VII collagen in the papillary dermal layer.
Forms bullae on acral skin ( finger tips, knuckles, elbows, knees, buttocks, toes, heels, ears)

17

Treatment for Epidermolysis Bullosa Acquisita

Systemic Corticosteroids, cyclosporine, colchicine, rituximab

18

Pathogensis of Linear IgA disease

IgA antibodies directed against a malformed skin protein (usually truncated BPAg2)

19

Most common trigger for Linear IgA disease

Vancomycin use

20

Treatment for Linear IgA disease

Stop using vanco
Dapsone

21

Pathogenesis of Dermatitis Herpetiformis

Skin manifestation of Celiac Sprue (gluten sensitive enteropathy)

22

Most common location of vesicle lesions of Dermatitis Herpetiformis

Extensor surfaces

23

First line treatment for Dermatitis Herpetiformis

Initiate Dapsone with a gluten free diet

24

Pathogenesis of Porphyria Cutanea Tarda

Defect in uroporphyrinogen decarboxylase
-blisters appear on the dorsum of the hands